多中心型Castleman病治疗进展

Castleman病(Castleman’s disease,CD)是一种少见的、病因不明的淋巴组织增生性疾病,1956年Castleman首先描述,临床表现多样化,按病变范围分为局灶型(1ocal Castleman’s disease,LCD)和多中心型(multicentric Castlemans disease,MCD),组织     

以发热、肾损伤和淋巴结肿大为主要表现的Castleman病1例

Castleman病临床表现不典型,极易漏诊误诊,加深对Castleman病临床病理特点的认识,是减少误诊的关键。本文讨论1例多中心型Castleman病,临床表现主要为发热、肾损伤、淋巴结肿大,入院后经临床-实验室检查-病理相结合,最终依据病理明确诊断。希望通过本病例,提高临床医师对该病的认识及诊断水平。     

副肿瘤性天疱疮伴发Castleman＇s病和间质性肺炎

目的总结首例伴发Castleman＇s病和间质性肺炎的副肿瘤性天疱疮（PNP）的诊治经验。方法结合患者临床表现和组织病理、免疫病理和影像学检查结果诊断为PNP伴Castleman＇s病和间质性肺炎。用大剂量丙种球蛋白中和抗体,手术切除肿瘤,用大剂量激素冲击疗法治疗间质性肺炎。结果患者临床症状均缓解,随访至今无复发。结论PNP不但与内脏肿瘤相关,也可累及呼吸系统;手术切除、大剂量丙种球蛋白及激素冲击疗法是该病的有效治疗方法。     

以肾病综合征为首发症状的多中心型Castleman病一例

Castleman病又称血管滤泡性淋巴组织增生或巨大淋巴结增生，是一种少见的慢性淋巴组织不典型增生性疾患，而多中心型Castleman病（浆细胞型）更为罕见。     

胰腺结核误诊为胰腺癌1例

结核累及胰腺非常罕见，临床症状有时不典型，术前诊断困难，现将中山医院收治的1例术前误诊为胰腺癌的胰腺结核报道如下。     

Caroli病合并多囊肾、胆总管狭窄1例报道

本文通过报道1例典型的Caroli病(Caroli’s disease,CD)合并多囊肾、胆总管狭窄(癌变可能)患者的病史资料及治疗回顾,并对比分析腹部CT、MRI、MRCP、ERCP等影像学检查,进一步探索该病的临床症状、诊断、鉴别以及治疗情况。     

伴骨髓毛细胞增多的Castleman病一例并文献复习

目的　报道 1例伴骨髓毛细胞增多的Castleman病 ,对其临床特征进行探讨并复习文献。方法与结果　贫血伴左下腹包块患者 ,经骨髓常规和电镜检查提示毛细胞白血病。常规干扰素治疗无效 ,切除左下腹包块 ,病检确诊为透明血管型Castleman病。术后随访 12年血象及骨髓象正常。结论　Castleman病临床表现多样化 ,易误诊。该病常伴有成熟浆细胞增多 ,伴典型毛细胞增多者尚未见报道。其确诊主要依靠病理学检查。本病预后良好 ,手术治疗后一般不复发     

多囊胰腺病的研究现状

多囊胰腺病是一类特殊的胰腺囊性疾病, 临床上非常罕见, 影像学上表现为胰腺实质呈多囊样改变。根据是否合并有其他脏器病变, 多囊胰腺病主要分为两类, 一类无胰腺外器官累及, 也称为孤立性多囊胰腺病;另一类属于特定类型遗传性疾病的表现之一。本文围绕多囊胰腺病的认识历程及孤立性多囊胰腺病的发病机制、影像学检查方法和临床预后等做一综述, 旨在加深对这一罕见疾病的认识。     

Castleman病并肺通气功能障碍1例并文献复习华中科技大学同济医学院附属同济医院 赵雅娟 赵建平 倪望＊,武汉 430030

目的：探讨胸部Castleman病的病因、临床特点、诊断和治疗方法,提高对该病的认识。方法：对1例纵隔局灶型Castleman病例回顾并结合文献进行复习。结果：本例患者表现为胸背痛,胸部CT发现左后纵隔占位,手术完整切除病灶后出现呼吸困难,肺功能提示极重度混合性肺通气功能障碍,病理活检明确Castleman 病(透明血管型)。予以激素、氧疗及对症支持治疗,病情无明显缓解。结论：局灶型Castleman病相关的弥漫性实质性肺疾病罕见,病理活检结合影像学表现可明确诊断,激素治疗效果有限。     

成人Still’s病一例报道并文献复习

成人Still’s（Adult—onset Still’s disease,AOSD）病是一组病因、发病机制尚不清楚,临床表现复杂多变或症状不典型,实验室检查又无特异性诊断指标的以发热、皮疹、关节痛为主要表现的临床综合征。在临床上确诊难度大,极易延误诊断及治疗,我们在临床上诊治1例,现报道如下。     

IgG4相关疾病误诊为多中心型Castleman病3例并文献回顾

目的通过病例资料分析IgG4相关疾病特点,总结IgG4相关疾病被误诊为多中心型Castleman病的原因,提高对此类疾病的诊断准确性。方法回顾性分析2008年1月至2012年12月北京协和医院诊治的3例IgG4相关疾病患者误诊为多中心型Castleman病的临床资料。结果 3例患者均为男性,年龄为53～57岁,均有全身广泛的淋巴结肿大及多系统受累,均曾行1次以上淋巴结活检。经病理检查,2例确诊、1例疑诊为多中心型Castleman病。筛查3例患者血清IgG4水平均明显升高,均大于1350mg/L;正电子发射计算机断层显像仪-CT(positron-emission tomography/computed tomography,PET-CT)显示全身广泛淋巴结及多脏器摄取增高;2例患者病理免疫组化证实IgG4阳性淋巴细胞占淋巴细胞50%以上。3例患者最终被确诊为IgG4相关疾病。经足量糖皮质激素及环磷酰胺治疗后3例患者的临床、血清学及影像学异常均迅速显著缓解。结论 IgG4相关疾病易被误诊为多中心型Castleman病,对临床表现、血清IgG4水平、PET-CT检查及病理免疫组化的综合分析有助于IgG4相关疾病的诊断。     

Castleman’s disease arising from the gallbladder neck causing difficulty in the differential diagnosis

We report a patient with Castleman’s disease arising from the gallbladder neck, which caused difficulty in making the differential diagnosis against gallbladder malignancies. A 50-year-old woman presented to our institution with epigastric pain. An abdominal computed tomography scan (CT) and magnetic resonance cholangiopancreatography (MRCP) study showed a 20-mm tumor located in the gallbladder neck for which malignancy could not be completely ruled out. For the definitive diagnosis and treatment, cholecystectomy was performed. In the operation, the main tumor and resection margins of the cystic duct were submitted for frozen section. The tumor was composed of a proliferation of lymphoid tissue with no signs of dysplasia. The ductal margin was free of tumor. The final histopathological diagnosis was unicentric Castleman’s disease, a hyaline vascular variant that developed in the gallbladder. The patient is currently in good condition without any signs of recurrence 28 months after the operation. This is the first detailed report of Castleman’s disease of the gallbladder. Making a correct diagnosis was very difficult before the operation, and only a surgical approach enabled confirmation of the diagnosis for this patient.     

Husten, Atemnot und B-Symptome bei einer 40-jährigen Frau

Multicentric Castleman’s disease (MCD) is a rare polyclonal lymphoproliferative disorder that is typically accompanied by an overproduction of circulating cytokines (mainly interleukin-6). We here report the case of a 40-year-old HIV-negative woman with pulmonary manifestation of MCD. There is no standard treatment for MCD. In our patient, various treatment courses with immunomodulatory drugs were unsuccessful. Finally, treatment with the interleukin-6 receptor antibody tocilizumab has resulted in continual clinical improvement over the last 5 years.     

Castleman病和副肿瘤性天疱疮相关性闭塞性细支气管炎

目的提高对Castleman病和副肿瘤性天疱疮（PNP）相关性闭塞性细支气管炎（OB）的认识。方法对1例Castleman病合并PNP和OB患者的临床资料进行分析,并结合相关文献进行复习。结果患者女性,54岁,主因“口腔溃疡3个月,咳嗽2个月,加重伴气短1个月”而入我院。肺功能显示严重的阻塞性通气功能障碍,支气管舒张试验阴性,肺弥散功能正常。x线胸片未见异常,胸部HRCT显示两肺密度不均匀,呈“马赛克样”分布。腹部CT显示盆腔腹膜后肿物,增强扫描肿物明显强化。口腔黏膜活检诊断为PNP,气管镜透壁肺活检显示细支气管管壁瘢痕组织形成,管腔狭窄及闭塞,诊断为0B;盆腔肿物手术切除后病理诊断为透明血管型Castleman病。在糖皮质激素治疗的基础上,予阿奇霉素口服、布地奈德／福莫特罗和噻托溴铵吸入剂等治疗。随访2年患者病情稳定。结论OB是伴有PNP的Castleman病的少见而严重的合并症,早期完全切除肿瘤、长期应用糖皮质激素、长效支气管扩张剂及大环内酯类药物等综合治疗将有助于改善预后。     

胰腺囊性病变的临床特征和诊治分析

背景:随着影像学技术的进步,胰腺囊性病变的检出率逐年升高,但鉴别不同类型的胰腺囊性病变仍是临床的棘手问题。目的:分析胰腺囊性病变的临床特征和诊治方法。方法:纳入2003年1月~2013年1月上海交通大学附属第一人民医院收治的胰腺囊性病变患者,对入组患者的临床表现、实验室检查、影像学特征以及治疗方法进行回顾性分析。结果:共49例患者纳入研究,其中假性囊肿13例、潴留囊肿4例、先天性囊肿2例、浆液性囊性肿瘤(SCN)9例、黏液性囊性肿瘤(MCN)11例、导管内乳头状黏液性肿瘤(IPMN)7例、实性-假乳头状肿瘤(SPN)3例。49例患者中16例患者无症状,其余33例患者表现为腹痛、腹胀、恶心、呕吐、发热等。13例胰腺假性囊肿患者均有急、慢性胰腺炎或外伤病史,3例IPMN患者血清CA19-9升高。B超、CT、MRI/MRCP、EUS诊断胰腺囊性病变的准确率分别为24.5%、32.7%、61.1%、100%。49例患者均接受手术治疗,分别行内引流术、囊肿切除术、胰十二指肠切除术、胰体(尾)切除术以及节段性胰腺切除术。结论:胰腺囊性病变无特异性临床表现。CT、MRI/MRCP作为无创检查手段,应广泛用于囊性病变的诊断和评估,EUS可作为进一步检查手段。选择合理的手术方案是治疗胰腺囊性病变的关键。     

The efficacy of tocilizumab in a patient with pulmonary arterial hypertension associated with Castleman’s disease

Castleman’s disease is a highly heterogeneous clinical-pathological entity that belongs to the lymphoproliferative disorders and is associated with pulmonary arterial hypertension (PAH) in some patients. It is linked to excessive immune stimulation by interleukin-6 (IL-6), which is also involved in the pathogenesis of PAH. A 31-year-old woman with Castleman’s disease demonstrated PAH characterized by severe right heart failure. Since she was resistant to various conventional therapies including steroids, prostacyclins, bosentan, and sildenafil, tocilizumab (anti-IL-6 receptor antibody) therapy was started. Her clinical course was followed for 6 months, with significant improvement without any adverse effect. This is the first reported case of use of tocilizumab in addition to steroids and conventional PAH therapy in a patient with PAH associated with Castleman’s disease.     

A rare case of hyaline-type Castleman disease in the liver

Castleman disease often develops in the neck, mediastinum and pulmonary hilum. Its onset in the peritoneal cavity is very rare. The patient, a woman in her 70s, was referred to our department for a detailed examination of an abdominal mass. On abdominal ultrasonography, computed tomography scan, magnetic resonance imaging and positron emission tomography, a mass approximately 15 mm in diameter was noted in the hepatic S6. We attempted radical treatment and conducted a laparoscope-assisted right lobectomy. On the basis of histopathological findings, the patient was diagnosed as having hyaline type Castleman disease in the liver, a very rare condition.     

巨大淋巴结增生症27例临床分析

目的提高对巨大淋巴结增生症(CD)的认识。方法收集中南大学湘雅二医院1996年1月至2008年5月27例经病理学确诊的CD患者资料,分为局灶(LCD)组和多中心(MCD)组。从临床表现、病理分型、治疗等方面进行回顾性分析和总结。结果 CD的严重程度和对治疗的反应与临床和病理学分型相关,MCD和浆细胞型往往预后较差。结论 LCD和MCD是拥有不同临床表现和预后的同一疾病的两种不同类型,其在发病机制和伴发疾病上有所差异。诊断时区分亚型是十分必要的。     

Endotracheal castleman disease: A case report

Castleman disease (CD) is an uncommon benign lymphoid hyperplasia with several clinical and morphologic variants associated with distinct outcomes. Pulmonary CD has been reported as a rare extranodal manifestation in the literature. However, CD presenting as an obstructive mass in the airway has not been documented. We report a case of localized hyaline-vascular CD presenting as an endotracheal lesion. The patient was a 50-year-old woman with Marfan syndrome. The lesion caused near-complete airway obstruction with respiratory insufficiency. The patient underwent laser resection, and the diagnosis of CD was supported by comprehensive studies including histopathologic, immunohistochemical, and molecular methods.